We report a 68-year-old Japanese female patient with subepidermal blistering disease with autoantibodies to multiple laminins, who subsequently developed membranous glomerulonephropathy. At skin disease stage, immunofluorescence demonstrated IgG anti-basement membrane zone antibodies reactive with dermal side of NaCl-split skin. Immunoblotting of human dermal extract, purified laminin-332, hemidesmosome-rich fraction and laminin-521 trimer recombinant protein (RP) detected laminin γ-1 and α-3 and γ-2 subunits of laminin-332. Three years after skin lesions disappeared, nephrotic symptoms developed. Antibodies to α-3 chain of type IV collagen (COL4A3) were negative, thus excluding the diagnosis of Goodpasture syndrome. All anti-laminin antibodies disappeared. Additional IB and ELISA studies of RPs of various COL4 chains revealed reactivity with COL4A5, but not with COL4A6 or COL4A3. Although diagnosis of anti-laminin γ-1 (p200) pemphigoid or anti-laminin-332-type mucous membrane pemphigoid could not be made, this case was similar to previous cases with autoantibodies to COL4A5 and/or COL4A6.

译文

:我们报道了一名68岁的日本女性,患有表皮下水疱病,对多种层粘连蛋白具有自身抗体,随后发展成膜性肾小球肾病。在皮肤疾病阶段,免疫荧光显示IgG抗基底膜区抗体可与NaCl分裂的皮肤的真皮侧发生反应。人类皮肤提取物,纯化的层粘连蛋白332,富含半脂质体的级分和层粘连蛋白521三聚体重组蛋白(RP)的免疫印迹检测到层粘连蛋白332的层粘连蛋白γ-1和α-3和γ-2亚基。皮肤病变消失三年后,出现肾病症状。 IV型胶原的α-3链抗体(COL4A3)呈阴性,因此排除了Goodpasture综合征的诊断。所有抗层粘连蛋白抗体均消失。对各种COL4链的RP进行的其他IB和ELISA研究表明,它与COL4A5有反应性,但与COL4A6或COL4A3没有反应性。尽管无法诊断出抗lamininγ-1(p200)天疱疮或抗laminin-332型粘膜天疱疮,但该病例与先前具有COL4A5和/或COL4A6自身抗体的病例相似。

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